Description

A congenital disorder involving the brain, skin and eyes. It is characterized by portwine nevi on upper part of the scalp along the distribution of the trigeminal nerve, as well as other vascular abnormalities both intracranially and in other parts of the body. Accumulations of abnormal blood vessels (angiomas) occur in the meninges of the cerebral cortex, usually on one side of the brain. Also choroid, intracranial calcifications, mental retardation, epileptic seizures, and glaucoma. Both sexes affected; present from birth. Inheritance, if any, is uncertain.

William Allen Sturge first described the syndrome in 1879 in a child aged 6 and a half years. Siegfried Kalischer in 1901, and Lannois-Bernoud in 1898 gave further descriptions. Parkes Weber in 1922 demonstrated the intracranial calcifications, Vincente Dimitri in 1923, and Krabbe in 1934 each contributed to the knowledge of the complete syndrome.

Lawford's syndrome is a variant with glaucoma, without increased ocular pressure. The term Schirmer's syndrome is used to indicate the asociation of early glaucoma (hydrophthalmia) and Sturge-Weber syndrome.

Right-sided hemi-hypertrophy resulting from right-sided congenital spastic hemiplegia, with a morbid condition of the left side of the brain, revealed by radiograms. Journal of Neurology and Psychopathology, London, 1922, 3: 134-139.

K. H. Krabbe:Facial and meningeal angiomatosis associated with calcification of the brain cortex. A clinical and an anatomopathologic contribution.Archives of Neurology and Psychiatry, Chicago, 1934, 32: 737-755.

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